Surgery of the hypertrophic cardiomyopathy remains controversial. The safest procedure and the simples procedure is the mitral valve replacement. Septal myomectomy is difficult because of the poor surgical exposure and complications: incomplete resection, complete heart block, ventricular septal defect. METHODS A 56 years old man with hypertrophic cardiomyopathy, double coronary stenosis and grade II mitral regurgitation by systolic anterior motion was long time stable under treatment with b-blockers. He developed angina and the circumflex and the left anterior descending arteries were stented. Re-stenosis developed in the left anterior descending stent and the patient was referred to surgery. The intraventricular gradient was 80 mmHg and the maximal septal thickness 28 mm. He was successfully treated by septal myomectomy and bypass on the left anterior descending artery with the left internal thoracic artery. Perioperative transesophageal echography was used to establish the limits of the surgical resection and to control the remnant gradient. The patient is asymptomatic 6 months after the procedure, he has a 30 mmHg remnant gradient and a grade I mitral regurgitation. CONCLUSIONS Septal myomectomy is a safe alternative to mitral valve replacement for hypertrophic cardiomyopathy. This procedure must be guided by perioperative transesophageal echography to avoid incomplete resection.